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| Name | Class |
|---|---|
| University of South Alabama | OTHER |
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Systemic sclerosis (SSc, also known as scleroderma) is a disease characterized by fibrosis of the skin and organs, inflammation, and an abnormal endothelial cell lining inside of vessels. A common and deadly complication of SSc is pulmonary hypertension (PH), which is an abnormal elevation in the blood pressure within the lung blood vessels. Early identification and treatment of PH is important in SSc, and no clinical factors can predict which patients will develop PH with acceptable accuracy. A potential marker of PH in SSc is the presence of increased amounts of endothelial microparticles (EMPs), which are substances circulating in the blood that were released from damaged vessel wall endothelial lining. A main goal of this study is to investigate if there is a difference in EMP levels between SSc patients with and without PH. The investigators will also use human endothelial cells in a lab environment to test whether these EMPs isolated from SSc patients are actually causing damage to the vessel lining. Lastly, the investigators will investigate the potential benefit of a medication used after transplant, mycophenolate mofetil (MMF). This will be done by causing damage to isolated human endothelial cells and treating them with MMF. The main goal of this portion of our study is to see if EMP levels are reduced when cells are treated with MMF. Overall, the investigators anticipate the following outcomes of this study: 1) use EMP levels to differentiation patients with SSc who have PH from those without PH, 2) use EMPs to understand how endothelial damage occurs in SSc, and 3) use EMPs to help us develop new treatments for patients with vascular diseases.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Systemic sclerosis, no pulmonary hypertension | This group will be systemic sclerosis patients without pulmonary hypertension |
| |
| Systemic sclerosis/pulmonary hypertension | This group will be systemic sclerosis patients with pulmonary hypertension |
| |
| Healthy controls | These will be healthy age- and sex-matched controls |
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| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| No intervention given | Other | There is no intervention for this study |
|
| Measure | Description | Time Frame |
|---|---|---|
| Plasma VE-cadherin + endothelial microparticle levels | endothelial microparticles are expressed as microparticles per microliter of plasma. This is a cross-sectional analysis, so the measurements will be performed once, on study visit 1 | Baseline |
| Measure | Description | Time Frame |
|---|---|---|
| Plasma PECAM+ endothelial microparticles | endothelial microparticles are expressed as microparticles per microliter of plasma. This is a cross-sectional analysis, so the measurements will be performed once, on study visit 1 | Baseline |
| Plasma E-selectin + endothelial microparticles |
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For systemic sclerosis subjects:
Inclusion Criteria:
Exclusion Criteria:
For healthy control subjects:
Inclusion Criteria:
Exclusion criteria:
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There will be 3 groups: systemic sclerosis subjects with pulmonary hypertension, systemic sclerosis subjects without pulmonary hypertension, and healthy age- and sex-matched controls.
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| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| LSU Health Sciences Center | New Orleans | Louisiana | 70125 | United States |
| PubMed Identifier | Type | Citation | Retractions |
|---|---|---|---|
| 20339146 | Background | Tual-Chalot S, Guibert C, Muller B, Savineau JP, Andriantsitohaina R, Martinez MC. Circulating microparticles from pulmonary hypertensive rats induce endothelial dysfunction. Am J Respir Crit Care Med. 2010 Jul 15;182(2):261-8. doi: 10.1164/rccm.200909-1347OC. Epub 2010 Mar 25. | |
| 18759303 | Background | Guiducci S, Distler JH, Jungel A, Huscher D, Huber LC, Michel BA, Gay RE, Pisetsky DS, Gay S, Matucci-Cerinic M, Distler O. The relationship between plasma microparticles and disease manifestations in patients with systemic sclerosis. Arthritis Rheum. 2008 Sep;58(9):2845-53. doi: 10.1002/art.23735. |
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| ID | Term |
|---|---|
| D012595 | Scleroderma, Systemic |
| D006976 | Hypertension, Pulmonary |
| D045743 | Scleroderma, Diffuse |
| ID | Term |
|---|---|
| D003240 | Connective Tissue Diseases |
| D017437 | Skin and Connective Tissue Diseases |
| D012871 | Skin Diseases |
| D008171 | Lung Diseases |
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Blood will be retained.
endothelial microparticles are expressed as microparticles per microliter of plasma. This is a cross-sectional analysis, so the measurements will be performed once, on study visit 1 |
| Baseline |
| 24016306 | Background | Iversen LV, Ostergaard O, Ullman S, Nielsen CT, Halberg P, Karlsmark T, Heegaard NH, Jacobsen S. Circulating microparticles and plasma levels of soluble E- and P-selectins in patients with systemic sclerosis. Scand J Rheumatol. 2013;42(6):473-82. doi: 10.3109/03009742.2013.796403. Epub 2013 Sep 9. |
| 18310479 | Background | Amabile N, Heiss C, Real WM, Minasi P, McGlothlin D, Rame EJ, Grossman W, De Marco T, Yeghiazarians Y. Circulating endothelial microparticle levels predict hemodynamic severity of pulmonary hypertension. Am J Respir Crit Care Med. 2008 Jun 1;177(11):1268-75. doi: 10.1164/rccm.200710-1458OC. Epub 2008 Feb 28. |
| 19782291 | Background | Amabile N, Heiss C, Chang V, Angeli FS, Damon L, Rame EJ, McGlothlin D, Grossman W, De Marco T, Yeghiazarians Y. Increased CD62e(+) endothelial microparticle levels predict poor outcome in pulmonary hypertension patients. J Heart Lung Transplant. 2009 Oct;28(10):1081-6. doi: 10.1016/j.healun.2009.06.005. |
| 30747487 | Derived | Lammi MR, Saketkoo LA, Okpechi SC, Ghonim MA, Wyczechowska D, Bauer N, Pyakurel K, Saito S, deBoisblanc BP, Boulares AH. Microparticles in systemic sclerosis: Potential pro-inflammatory mediators and pulmonary hypertension biomarkers. Respirology. 2019 Jul;24(7):675-683. doi: 10.1111/resp.13500. Epub 2019 Feb 12. |
| D012140 | Respiratory Tract Diseases |
| D006973 | Hypertension |
| D014652 | Vascular Diseases |
| D002318 | Cardiovascular Diseases |